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货号: PD5779
货期: 现货
价格: 50μL/960;100μL/1600;200μL/2560
所属分类:
靶点: Factor I
应用: WB,ELISA
反应性 : Human,Rat,Mouse,
宿主物种 : Rabbit
MW(Observed): Full lenth:66kD,heavy chain:50-58kD
同种型-Isotype : IgG
隐藏域元素占位
详细信息
推荐稀释比 : WB 1:500-2000;ELISA 1:10000;Not yet tested in other applications.
组成 : Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺 : The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
储存 : -15°C to -25°C/1 year(Do not lower than -25°C
浓度 : 1 mg/ml
克隆性 : Polyclonal
抗原&靶点信息
特异性 : Factor I Polyclonal Antibody detects endogenous levels of Factor I protein.
基因名称 : CFI
蛋白名称 : Complement factor I
别名 : CFI;IF;Complement factor I;C3B/C4B inactivator
背景:
This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by Ref


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